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STEVENS JOHNSONS TEN
Spectrum:
Erythema Multiforme
Stevens Johnsons
TEN
Several classifications:
SJS - A "minor form of TEN," with less than 10% body surface area (BSA)
detachment
Overlapping SJS/TEN - Detachment of 10-30% BSA
TEN - Detachment of more than 30% BSA
Aetiology
Immune complex mediated hypersensitivity
Serious systemic disorder  potential for severe morbidity & death
Ie Multi-system disease
Skin & mucus membranes involved
Mucus membs affected:
Oral
Nasal
Eye
Vaginal
Urethral
GIT
) can progress to necrosis
Lower Resp Tract
)
Complications
Ophthalmologic - Corneal ulceration, anterior uveitis, panophthalmitis, blindness
Gastroenterologic - Esophageal strictures
Genitourinary - Renal tubular necrosis, renal failure, penile scarring, vaginal stenosis
Pulmonary - Tracheobronchial shedding with resultant respiratory failure
Cutaneous - Scarring and cosmetic deformity, recurrences of infection through slowhealing ulcerations
Prognosis
Individual lesions typically should heal within 1-2 weeks, unless secondary infection
occurs.
Most patients recover without sequelae.
Development of serious sequelae, such as respiratory failure, renal failure, and
blindness, determines prognosis in those affected.
Up to 15% of all patients with SJS die as a result of the condition.
The SCORTEN score looks at a number of variables and uses them to prognosticate
risk factors for death in both SJS and TEN.
Mortality
Determined primarily by extent of skin sloughing
BSA sloughing < 10% mortality = approx1-5%
BSA sloughing > 30% mortality = 25%-35%.
Morbidity
Lesions can erupt in crops for 2-3 weeks
Mucosal pseudomembrane formation can lead to mucosal scarring and loss of
function of affected organ
Esophageal strictures if extensive involvement of the esophagus
Mucosal shedding in the tracheobronchial tree may lead to respiratory failure.
Ocular sequelae may include corneal ulceration and anterior uveitis. Blindness may
develop secondary to severe keratitis or panophthalmitis in 3-10% of patients.
Vaginal stenosis and penile scarring have been reported
Renal complications are rare.
History
Typically, the disease process begins with a non-specific upper respiratory tract
infection.
Prodrome:
1-14days, URTI like Syx
fever, sore throat, chills, headache, and malaise
Occasionally vomiting and diarrhoea
Mucocutaneous lesions develop abruptly.
Clusters of outbreaks last from 2-4 weeks.
The lesions are typically nonpruritic.
A history of fever or localized worsening should suggest a superimposed infection
(but fever reported in up to 85% of cases)
Involvement of oral and/or mucous membranes may be severe enough that patients
may not be able to eat or drink
Genitourinary involvement dysuria or an inability to void
A history of a previous outbreak of Stevens-Johnson syndrome (SJS) or of erythema
multiforme may be elicited.
Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.
Typical symptoms are as follows:
o Cough productive of a thick purulent sputum
o Headache
o Malaise
o Arthralgia
Physical
Rash can begin as macules that develop into papules, vesicles, bullae, urticarial
plaques, or confluent erythema.
o The center of these lesions may be vesicular, purpuric, or necrotic.
o The typical lesion has the appearance of a target.
The target is considered pathognomonic.
However, in contrast to the typical erythema multiforme lesions, these lesions have
only two zones of color.
The core may be vesicular, purpuric, or necrotic; that zone is surrounded by macular
erythema.
Some have called these targetoid lesions.
o Lesions may become bullous and later rupture, leaving denuded skin. The
skin becomes susceptible to secondary infection.
o Urticarial lesions typically are not pruritic.
o Infection may be responsible for the scarring associated with morbidity.
o Although lesions may occur anywhere, the palms, soles, dorsum of the hands,
and extensor surfaces are most commonly affected.
o The rash may be confined to any one area of the body, most often the trunk.
o Mucosal involvement may include erythema, edema, sloughing, blistering,
ulceration, and necrosis.
o Although some have suggested the possibility of SJS without skin lesions,
most believe that mucosal lesions alone are not enough to establish the
diagnosis.
The following signs may be noted on examination:
o Fever
o Orthostasis
o Tachycardia
o Hypotension
o Altered level of consciousness
o Epistaxis
o Conjunctivitis
o Corneal ulcerations
o Erosive vulvovaginitis or balanitis
o Seizures, coma
Causes
Drugs and malignancies are most often implicated as the etiology in adults/elderly
* Pediatric cases are related more often to infections than to malignancy or a
reaction to a drug.
* A medication such as sulfa, phenytoin, or penicillin had previously been
prescribed to more than two thirds of all patients with SJS.
The anticonvulsant oxcarbazepine (Trileptal) has also been implicated.
Reported in HIV patients treated with nevirapine, may extend to other non-nucleoside
reverse transcriptase inhibitors.
Indinavir has been mentioned.
In 2007, the FDA issued a warning that SJS/TEN had occurred in patients taking
modafinil (Provigil).
More than half of the patients with SJS report a recent upper respiratory tract
infection.
* The 4 etiologic categories are
(1) infectious
(2) drug-induced
(3) malignancy-related
(4) idiopathic
o Viral diseases that have been reported include herpes simplex virus (HSV),
AIDS, coxsackie viral infections, influenza, hepatitis, mumps, mycoplasmal infection,
lymphogranuloma venereum (LGV), rickettsial infections, and variola.
o Bacterial etiologies include group A beta streptococci, diphtheria, Brucellosis,
mycobacteria, Mycoplasma pneumoniae, tularemia, and typhoid.
o Coccidioidomycosis, dermatophytosis, and histoplasmosis are the fungal
possibilities.
o Malaria and trichomoniasis have been reported as protozoal causes.
o In children, Epstein-Barr virus and enteroviruses have been identified.
o Drug etiologies include penicillins and sulfa antibiotics. Anticonvulsants
including phenytoin, carbamazepine, valproic acid, lamotrigine, and barbiturates have
been implicated. Mockenhapupt et al stressed that most anticonvulsant-induced SJS
occurs in the first 60 days of use.4 In late 2002, the US Food and Drug
Administration (FDA) and the manufacturer Pharmacia noted that SJS had been
reported in patients taking the cyclooxygenase-2 (COX-2) inhibitor valdecoxib. In
2007, the US FDA reported SJS/TEN in patients taking modafinil (Provigil).
o Various carcinomas and lymphomas have been associated.
o SJS is idiopathic in 25-50% of cases.
DIAGNOSIS
Skin biopsy is the definitive diagnostic study but is not an emergency department
(ED) procedure.
o Skin biopsy specimens demonstrate that the bullae are subepidermal.
o Epidermal cell necrosis may be noted.
o Perivascular areas are infiltrated with lymphocytes.
ED MANAGEMENT
The single most important role for the ED physician is to detect SJS/TEN early and
initiate the appropriate ED and inpatient management.
Withdrawal of the suspected offending agent is critically important. Timing of
withdrawal has been linked to outcome.
* Care in the ED must be directed to fluid replacement and electrolyte correction.
* Skin lesions are treated as burns.
* Patients with SJS/TEN should be treated with special attention to airway and
hemodynamic stability, fluid status, wound/burn care, and pain control.
* Treatment is primarily supportive and symptomatic. Some have advocated
cyclophosphamide, plasmapheresis, hemodialysis, and immunoglobulin. Most
authorities believe that corticosteroids are contraindicated.
*
o Manage oral lesions with mouthwashes.
o Topical anesthetics are useful in reducing pain and allowing the patient to
take in fluids.
o Areas of denuded skin must be covered with compresses of saline or Burow
solution.
* Underlying diseases and secondary infections must be identified and treated.
Offending drugs must be stopped.
* The use of systemic steroids is controversial. Some authors believe that they are
contraindicated. Treatment with systemic steroids has been associated with an
increased prevalence of complications.
* Address tetanus prophylaxis.
Consultations
Consultants may help establish the diagnosis and direct inpatient care. A
dermatologist is the most likely clinician to establish the diagnosis, with or without
biopsy.
* Severe cases may require the involvement of a burn specialist or plastic surgery
specialist.
* Internal medicine, critical care, or pediatrics consultants direct inpatient care.
* Ophthalmology consultation is mandatory for those with ocular involvement.
* Depending on organ system involvement, consultations with a gastroenterologist,
pulmonologist, and nephrologist may be helpful.
ONGOING CARE
Careful daily inspection is necessary to monitor for secondary superinfections.
Although patients with erythema multiforme minor may be treated as outpatients with
topical steroids, those with erythema multiforme major (ie, SJS) must be hospitalized.
Cases of erythema multiforme minor must be followed closely. Some authors
recommend daily follow-up.